Lec 3

Question 1

What is the prophyrins?

Answer 1

Cyclic compound consist of 4 pyrrole ring connection by methine bridges

Question 2

What is the difference between porphyrins compounds?

Answer 2

Side chane and distribution of it

Question 3

What is heme ?

Answer 3

Protoporphyrin IX (one of porphyrine compounds) with FE

Question 4

Where biosynthesis of heme occurs ?

Answer 4

In liver (15%) & in erythrocytes (85%)

Question 5

What is the enzyme that shared in first step of heme biosynthesis?

Answer 5

Aminolevulinic acid synthase 1

Question 6

What is the precursor of aminolavulinic acid ?

Answer 6

Glycine & succinyl coA

Question 7

What is the products of first step of heme biosynthesis?

Answer 7

Aminolevulinic acid & coA & co2

Question 8

What is the rate limiting step in heme biosynthesis?

Answer 8

Aminolevulinic acid synthase

Question 9

Does heme affect ALAS1?

Answer 9

Yes inhibit it

Question 10

What is the enzyme that lead inhibit it in heme biosynthesis?

Answer 10

Aminolevulinic acid reductase & ferrochelatase

Question 11

What is the porphyria type I

Answer 11

Deficiency in utoporphyrinogen III synthase

Question 12

What is the enzyme that convert protoporphyrin IX to heme ?

Answer 12

Ferrochelatase enzyme which add FE to protoporphyrin

Question 13

Ferrochelatase enzyme found in ?

Answer 13

Mitochondria

Question 14

Aminolevulinic acid synthase found in ?

Answer 14

Moitochondia

Question 15

How I can detected the deficiency of FE?

Answer 15

By elevated the zinc protoporphyrin which zinc bind to protoporphyrin instead of FE

Question 16

What is the porphyria?

Answer 16

Porphyria refers to a group of disorders that result from a buildup of natural chemicals that
produce porphyrin in the body.

Question 17

What is the categories of porphyria?

Answer 17

Acute (affect CNS) cautaneous (affect the skin)

Question 18

Which one of porphyria is recessive ?

Answer 18

Congenital eythropoietic porphyria

Question 19

What is the result of prophyria?

Answer 19

Formation of superoxide radical , skin blister, skin darken

Question 20

What is the most common porphyria ?

Answer 20

Porphyria cutanea tarda

Question 21

What is the cause of porphyria cutanea tarda ?

Answer 21

Deficiency in uroporphyrinogin decarboxylase enzyme

Question 22

What is the function of uroporphyrinogen decarboxylase?

Answer 22

Convert uroporphyrin to coproporphyrinogen

Question 23

What is the result of porphyria cutanea tarda?

Answer 23

Acummulation of uroporphyrogen in urine which lead to converted the color of urine to red , and lead to red to brown skin eruptions

Question 24

What is the significant of heme degradation ?

Answer 24

Prevent heme toxicity
Excretory system
Product of degradation act as messenger products
Response to oxidative stress

Question 25

What is the enzyme that act on first step of heme degradation?

Answer 25

Heme oxygenation

Question 26

The first step of heme degradation need NADPH or NADH or FADH2?

Answer 26

NADPH

Question 27

What is the compound that result from first step of heme degradation?

Answer 27

Biliverdin & co & FE+3

Question 28

What is the enzyme that convert biliverdin to bilirubin?

Answer 28

Biliverdin reductase (need NADPH)

Question 29

Synthesis of bilirubin occurs where?

Answer 29

In macrophages

Question 30

What is the protein that bilirubin bind with it in blood

Answer 30

Albumins

Question 31

What is the conjugation form of bilirubin?

Answer 31

Bilirubin diglucuronide

Question 32

Which enzyme convert bilirubin to bilirubin diglucuronide

Answer 32

Bilirubin glucuronyl transferase (needed 2 glucornic acid)

Question 33

What is the color of bilirubin?

Answer 33

Red-orange

Question 34

What is the effect of salicylate & sulfonamide on bilirubin?

Answer 34

It replaces it in bilirubin which lead to enter CNS and neural damaged

Question 35

Deficiency in bilirubin glucurnyl transferase lead to ?

Answer 35

Gilbert syndrome & crigler najjar

Question 36

Deficiency in enzyme that transport conjugated bilirubin out the liver lead to

Answer 36

Dubin-Johnson syndrome

Question 37

How urobilinogen synthase ?

Answer 37

Bacteria reduced bilirubin diglucuronide by remove glucornic acid

Question 38

What is the compound that secreted to feces (final compound of bilirubin) and what is the color of it and how it production?

Answer 38

This compound is sterocobilin and produce by oxidized urobilinogen and have brown color

Question 39

Urobilinogen or sterocobilin secreted by urine and which one by feces?

Answer 39

Urobilinogen by iron & sterocobilin by feces

Question 40

What is the juandice ?

Answer 40

Yellow color of skin , nail bad & sclera

Question 41

He have 3 type of juandice, mention them and what is the type of bilirubin increase during this ?

Answer 41

1-prehepatic juandice, which lead to increase in unconjugated bilirubin 2-hepatocellural juandice, which lead to increased in both conjugated & unconjugated bilirubin 3-posthepatic or cholestatic juandice, which lead to increase in conjugated bilirubin

Question 42

What is the kernicterus?

Answer 42

Damaged to brain due to accumulation of bilirubin in brain

Question 43

By any type of bilirubin kernicterus can occurs ?

Answer 43

By unconjugated because it is water insoluble so it can bass B.B.B